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1 고립성우측폐동맥형성부전증 1 례 이달의 X 선 연세대학교의과대학내과학교실 1, 폐질환연구소 2, 진단방사선과학교실 3 김도연 1, 이재성 1, 김영 1, 장윤수 1, 김형중 1,2, 김태훈 3, 안철민 1,2 A Case of Isolated Right Pulmonary Artery Agenesis Do Youn Kim, M.D. 1, Jae Sung Lee, M.D. 1, Young Kim, M.D. 1, Yoon Soo Chang, M.D. 1, Hyung Jung Kim, M.D. 1,2, Tae Hoon Kim, 3, Chul Min Ahn, M.D. 1,2, Department of Internal Medicine 1, The institute of Chest Disease 2 and Department of Radiology 3, Yonsei University College of Medicine, Seoul, Korea Unilateral pulmonary artery agenesis is a rare congenital anomaly usually associated with other cardiovascular anomalies such as tetralogy of Fallot or septal defect. Unilateral pulmonary artery agenesis without other coexisting cardiovascular abnormality (isolated unilateral pulmonary artery) is extremely rare and often asymptomatic until adulthood. In these patients, diagnostic clue is found in a plain chest roentgenogram, showing a hyperlucent contracted hemithorax. We have recently experienced a case of isolated right pulmonary artery agenesis, which was diagnosed by chest dynamic CT, perfusion scan, echocardiogram and 3-dimensional reconstruction cardiac CT angiography in a 50-year old female who had suffered from mild dyspnea on exertion and improved with conservative treatment. We report this case with a brief review of the relevant literature. (Tuberc Respir Dis 2004; 57: ) Key words : Isolated right Pulmonary artery agenesis. 증례 환자 : 이 희, 여자, 50세주소 : 운동시호흡곤란현병력 : 환자는특이증상없이건강히지내던중내원 2개월전부터운동시간헐적인호흡곤란있어개인의원에내원하여치료받았으나증상호전없어본원호흡기내과로전원되어외래경유하여입원하였다. 과거력 : 특이사항없음진찰소견 : 내원시신체검사상혈압은 150/80mmHg, 맥박은분당 64회, 호흡수는분당 20회였고, 체온은 36.5 이었다. 외견상청색증등은없었고흉부청진상심잡음은없었으나우측폐야에서호흡음이경미하게감소되어있었다. Address for correspondence : Chul Min Ahn, M.D. Department of internal Medicine, Yonsei University College of Medicine , Dogok-Dong, Kangnam-Ku, Seoul , Korea Phone : Fax : Ahnswine@naver.com Received : Jul Accepted : Sep 검사소견 : 내원당시말초혈액검사는백혈구 8,280/mm 3 ( 중성구 62.2%), 혈색소 15.0g/dl, 혈소판 186,000/mm 3 이었으며동맥혈가스검사는 ph 7.392, PaO mmhg, PaCO mmhg, HCO mmol/l, O 2 sat 91.2% 로저산소혈증의소견을보였다. 단순폐기능검사상노력성폐활량 (FVC) 은 2.49L ( 정상예측치의 73%), 1초간노력성호기량 (FEV 1 ) 은 2.09L ( 정상예측치의 81%), FEV 1 /FVC는 83% 로경도의제한성폐기능장애소견을보였다. 방사선학적소견 : 단순흉부방사선검사상우측흉곽의크기가좌측흉곽의크기에비해작았으며, 우측폐는형성부전이있는반면좌측폐는과도하게발육되어있었고, 기관및종격동이경미하게우측으로전위되어있었다. 우측늑골간격이좁아져있었고우측폐문부혈관음영은감소되어있었으나양측폐야에국소적인병변은없었다 (Fig 1). 흉부전산화단층촬영에서는우측폐가작아져있었고, 우측폐혈관형성부전과우측늑간동맥의비대소견을보였다 (Fig 2. A). 삼차원재구성전산화심혈관단층촬영 (3-dimensional reconstruction cardiac CT angio graphy) 에서는폐동맥간에서분지되어좌측폐로가는좌측폐동맥은잘발육되어보이나우측폐동맥은 489

DY Kim, et al.: A case of isolated right pulmonary artery agenesis 은 27.7mmHg 로정상소견을보였다. 치료및경과 : 환자는입원이후고립성우측폐동맥형성부전증으로진단되어보존적치료후증상호전을보여, 외래에서추적관찰하기로하고퇴원하였다. 고찰 Figure 1.

A), 폐관류스캔에서는우측폐에폐동맥관류가전혀없는소견을보였다 (Fig 3. B). 심초음파검사상심박출계수는 56% 였고심기형은보이지않았으며, 우심실수축기압 편측폐동맥형성부전증은약 20만명당한명의발생률이있는비교적드문질환으로팔로사징 (tetralogy of Fallot) 등의선천성심기형을동반하는경우가많으며 Franzel 에의해 1868년처음보고되었다 1.

일측성폐동맥형성부전증은팔로사징 (tetralogy of Fallot), 동맥관개존증, 심실중격결손, 대동맥협착증, 폐동맥협착증, 대혈관전위, 우측대동맥궁, 대동맥폐동맥창, 전폐정맥연결이상, 타우시-빙기형 (Taus sig-bing malformation), Scimitar

2 DY Kim, et al.: A case of isolated right pulmonary artery agenesis 은 27.7mmHg 로정상소견을보였다. 치료및경과 : 환자는입원이후고립성우측폐동맥형성부전증으로진단되어보존적치료후증상호전을보여, 외래에서추적관찰하기로하고퇴원하였다. 고찰 Figure 1. Chest PA: Chest PA showed decreased size of right lung and mild tracheal deviation to right side and decreased right hilar vascular marking. 기시부에서부터형성부전의소견을보였다 (Fig 2. B). 폐환기스캔은정상소견을보였으나 (Fig 3. A), 폐관류스캔에서는우측폐에폐동맥관류가전혀없는소견을보였다 (Fig 3. B). 심초음파검사상심박출계수는 56% 였고심기형은보이지않았으며, 우심실수축기압 편측폐동맥형성부전증은약 20만명당한명의발생률이있는비교적드문질환으로팔로사징 (tetralogy of Fallot) 등의선천성심기형을동반하는경우가많으며 Franzel 에의해 1868년처음보고되었다 1. 또한타장기나선천성심기형이동반되지않은고립성편측폐동맥형성부전증은극히드물어 1952 년 Madoff등에의해처음으로문헌에보고되었고국내에서는 1991년김용환등에의해처음보고된바있다 2. 일측성폐동맥형성부전증은팔로사징 (tetralogy of Fallot), 동맥관개존증, 심실중격결손, 대동맥협착증, 폐동맥협착증, 대혈관전위, 우측대동맥궁, 대동맥폐동맥창, 전폐정맥연결이상, 타우시-빙기형 (Taus sig-bing malformation), Scimitar 증후군등의선천성심장기형을동반한경우가많으며증상발현또한빨라대개생후일년이내에진단되는경우가많다. 그러 Figure 2A. Chest CT : Chest CT showed decreased right lung volume without hypoplastic change, non-visualization of right pulmonary artery and tortous and dilatated right intercostal artery. Figure 2B. 3-Dimensional reconstruction cardiac CT angiography : 3D reconstruction CT showed interru ption of right pulmonary artery. 490

편측폐동맥형성부전증환자는반복적인폐감염, 경도의노작성호흡곤란, 객혈의증상을보이며, 진단되는환자의 37% 정도에서반복적인폐감염증상을보이는것으로알려져있다 1,4. 반복적인폐감염의병인은아직까지확실히밝혀진바는없으나폐포저탄산증에의한기관지수축과폐관류저하에의해서흡인미생물에대한염증세포의전달결손및점액섬모청소능의감소로이해되고있다 1,5.

3 Tuberculosis and Respiratory Diseases Vol. 57. No. 5, Nov, 2004 Figure 3A. Ventilation scan : Ventilation scan showed normal finding. 나심장기형을동반하지않은고립성편측폐동맥형성부전증인경우대부분무증상이며간혹반복적인폐감염, 객혈혹은경도의노작성호흡곤란의증상만을보여사오십대에진단되는경우도있다. 발생학적으로는근위폐동맥으로발전할여섯번째대동맥궁의근위부가퇴화되어폐외폐동맥형성부전이생기고폐내폐동맥과여섯번째대동맥의원위부의연결이지속되어폐내폐동맥형성부전증이발생하게된다 3. 편측폐동맥형성부전증환자는반복적인폐감염, 경도의노작성호흡곤란, 객혈의증상을보이며, 진단되는환자의 37% 정도에서반복적인폐감염증상을보이는것으로알려져있다 1,4. 반복적인폐감염의병인은아직까지확실히밝혀진바는없으나폐포저탄산증에의한기관지수축과폐관류저하에의해서흡인미생물에대한염증세포의전달결손및점액섬모청소능의감소로이해되고있다 1,5. 이러한반복적인폐감염으로인해일부환자에는기관지확장증이유발되기도한다. 운동시호흡곤란은운동시의일회호흡량에대한생리학적사강의비율이증가하는것을원인으로들수있다. 또한기관지동맥의산소분압이대동맥의산소분압과같아병변측폐포와모세혈관과의산소분압차가적어병변측폐에서교환되는산소량이무 Figure 3B. Lung perfusion scan : Lung perfusion scan using Tc-99m MAA showed perfusion defect (near absence of pulmonary arterial perfusion) on right lung. Measured right to left lung activity ratio was 2.3% to 97.7%, respectively. 시할만큼적은것도노작성호흡곤란의원인으로들수있다 6,7. 편측폐동맥형성부전증으로진단되는환자의 20% 에서는반복적인객혈을나타내는데폐동맥관류부전으로인해기관지동맥, 늑간동맥, 쇄골하동맥, 횡격막하동맥과같은체순환에의한측부순환이발달되어있으며, 특히정상적으로는기관지동맥으로심박출량의 1% 가관류되나폐동맥형성부전증에서는 17% 까지증가하여비대해지고사행성변화를보이는기관지동맥과측부순환의파열이폐출혈과객혈의원인이된다 1,8. 대개폐출혈은자연적으로멈추게되나다량의폐출혈이나지속적인출혈인경우색전술이나폐절제술이필요하기도하고이러한폐출혈이나폐고혈압이있을시장기생존율을저해하여약 7% 정도의사망률을보인다 1. 또한, 저산소혈증에대한폐혈관의민감도에차이가있어일부분의환자에서는해발 3000m 이상의고산지대에서는폐혈관수축에의한폐부종이나폐고혈압이더욱빈번하게발생하고저산지대로내려와산소치료및혈관확장제사용시가역적인호전을보이게된다 1,9,

4 DY Kim, et al.: A case of isolated right pulmonary artery agenesis 고립성편측폐동맥형성부전증환자는이학적검사상병변측호흡음이감소되어있으며단순흉부방사선촬영상병변측폐가작아져있다. 또한, 폐혈관음영이보이지않으며심장과종격동이병변측으로전위되어있고횡격막상승을보이게된다. 환기-관류스캔상환기스캔은정상이거나경도로감소되어있으며지연배출은보이지않으나관류스캔에서병변측관류의결손을보이는양상을보이는데종괴나급성증상이없는상태에서이러한소견을보인다면편측폐동맥형성부전증이나폐동맥협착증을의심할수있다 11. 역동적흉부전산화단층촬영에서는동반된선천성심기형유무및폐동맥을대신하여풍부하고비대해진측부순환을관찰할수있으며고해상전산화단층촬영에서는반복적인폐감염환자에서기관지확장증유무를확인할수있다. 심초음파소견으로는동반된심기형과폐고혈압을확인할수있으며이러한영상기술로인해수술을시행하지않을환자에게서심도자술의필요성은점차감소되고있다 1. Swyer James 증후군, 폐동맥혈전증, 폐동맥협착증, 폐결핵등이단순흉부방사선촬영에서유사한양상을보이나임상증상과이학적소견및혈관촬영등으로감별진단할수있으며특히, Swyer James 증후군은반복적인만성폐감염에의해병변측폐에이차적으로폐혈관변화가초래되고공기저류가생겨폐음영의과투과성을보이는질환으로편측폐동맥형성부전증과는달리환기스캔에서지연배출소견을보인다 1,7. 고립성편측폐동맥형성부전증의확진은환기-관류스캔이나혈관조영술을통하여이루어지며혈관조영술상일측의페동맥이조영되지않는것으로확진할수있는데, 본증례의경우삼차원재구성전산화심혈관단층촬영 (3-dimensional reconstruction cardiac CT angiography) 을이용하여비침습적인방법으로고립성편측폐동맥형성부전증을진단하였다. 삼차원재구성전산화심혈관단층촬영술은기존의폐혈관조영술에비해안전하고실행하기쉬우며어른뿐아니라어린이에서도선천성심기형이동반된일측성폐동맥형성부전증을진단하는데매우유용하 게사용될수있다. 치료는심한폐고혈압및폐부종동반시폐혈관재관류술을시행할수있으며반복적인감염이나심한기관지확장증및반복적인심한각혈이있을때는폐절제술도고려할수있다. 또한재관류후에도폐고혈압이교정이되지않는경우원발성폐고혈압의동반을의심하여야하며원발성폐고혈압이나선천성심기형에의한이차성폐고혈압이진단되면구강칼슘차단제나프로스타사이클린정맥주사가도움이된다 1. 그리고본례와같이선천성심기형을동반하지않고비교적증상이경미하며성인이되어진단되는경우에는보존적치료를시행할수있다. 요약 일측성폐동맥형성부전증은매우드문선천성기형으로서대부분팔로사징, 심실중격결손등과같은선천성심기형을동반하며선천성심기형이동반되지않은고립성편측폐동맥형성부전증은극히드물다. 이러한환자들의경우성인이될때까지무증상으로지내는경우가많으며흉부방사선검사상병변측폐의폐음영은과투과되어나타나며흉곽은작아진소견으로부터고립성편측폐동맥형성부전증을의심하게된다. 저자들은경미한운동시호흡곤란을증상으로내원한 50세여환에서흉부전산화단층촬영및환기-관류스캔과심초음파검사를시행하고, 삼차원재구성전산화심혈관단층촬영을이용하여고립성우측폐동맥형성부전증을진단하고치료한 1례를경험하였기에문헌고찰과함께보고하는바이다. 참고문헌 1. Ten Harkel AD, Blom NA, Offenkamp J. Isolated unilateral absence of a pulmonary artery: A Case Report and Review of the Literature. Chest 2002; 122: Kim YH, Jo KH, Kwack MS, Kim SW, Lee HK. Left pulmonary artery agenesis. Kor J Thoracic Cardio vasc surgery 1991;24:

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